Endocrinology focuses on hormonal system and metabolic health. Learn about the diagnosis and treatment of diabetes, thyroid disorders, and adrenal conditions.
Send us all your questions or requests, and our expert team will assist you.
The definitive treatment for pheochromocytoma is the surgical removal of the tumor. However, the journey to surgery is complex and requires meticulous medical optimization to ensure patient safety. Unlike other surgeries where a patient can proceed directly to the operating room, operating on a patient with an active pheochromocytoma without preparation is perilous. The induction of anesthesia or manipulation of the tumor can trigger a massive release of catecholamines, leading to a hypertensive crisis, arrhythmia, or stroke. Therefore, a strict protocol of preoperative medical management is mandatory. Following surgery, management shifts to recovery, monitoring for complications, and long-term surveillance. This section outlines the comprehensive care pathway from preoperative stabilization to surgical intervention and postoperative follow-up.
The primary goal of preoperative management is to block the effects of the excess catecholamines, thereby normalizing blood pressure and heart rate and expanding the contracted blood volume. This phase usually lasts for 7 to 14 days before the scheduled surgery. The cornerstone of this preparation is alpha-adrenergic blockade. By blocking the alpha receptors on blood vessels, the medication prevents the vasoconstriction caused by norepinephrine. Once adequate alpha-blockade is achieved, beta-blockade may be added if necessary to control heart rate.
Phenoxybenzamine is the most commonly used non-selective alpha-blocker for this purpose. It is started at a low dose and gradually titrated up until blood pressure is controlled and the patient experiences mild orthostatic symptoms, which indicates effective vasodilation. Selective alpha-1 blockers like doxazosin or prazosin are alternatives that may have fewer side effects but require careful dosing. The goal is to protect the patient from the hypertensive surges that can occur during tumor handling. Side effects like nasal congestion and fatigue are common but indicate that the drug is working.
Beta-blockers are used to manage tachycardia (fast heart rate) that can result from the excess epinephrine or as a reflex response to alpha-blockade. However, it is a cardinal rule in pheochromocytoma management that beta-blockers must never be started before adequate alpha-blockade has been established. Blocking beta receptors alone leaves the alpha receptors unopposed, which can lead to a paradoxical and dangerous rise in blood pressure due to unopposed vasoconstriction. Once alpha-blockade is in place, beta-blockers like propranolol or metoprolol can be safely introduced to control the heart rate.
Adrenalectomy, the surgical removal of the adrenal gland, is the definitive cure for sporadic pheochromocytoma. The choice of surgical technique depends on the size of the tumor, the suspicion of malignancy, and the surgeon’s expertise. Modern surgical practices favor minimally invasive techniques whenever possible, as they offer faster recovery and less postoperative pain. However, the fundamental principles of surgery remain the same: minimal manipulation of the tumor to prevent hormone release and complete resection of the mass.
Laparoscopic (keyhole) adrenalectomy is the gold standard for most pheochromocytomas that are less than 6 to 8 centimeters in size and do not show signs of local invasion. This approach involves making small incisions in the abdomen to insert a camera and instruments. Another option is the posterior retroperitoneoscopic adrenalectomy, where the approach is from the back. This technique avoids entering the abdominal cavity, reducing the risk of injury to internal organs and preventing scar tissue formation. Minimally invasive surgery results in shorter hospital stays and a quicker return to normal activities.
Open surgery is reserved for large tumors (typically larger than 8-10 cm), those with signs of invasion into surrounding organs, or when there is a high probability of malignancy. An open approach allows the surgeon to visualize the entire operative field, safely dissect the tumor from major blood vessels like the vena cava, and perform a wide resection to ensure negative margins. While the recovery is longer compared to laparoscopic surgery, the safety and completeness of the cancer resection take precedence in these complex cases.
Anesthesia for pheochromocytoma resection is a high-stakes endeavor requiring an experienced anesthesiology team. Throughout the surgery, the patient’s blood pressure can fluctuate wildly. During the dissection phase, manipulation of the tumor can cause hypertensive crises requiring immediate administration of rapid-acting vasodilators. Conversely, once the tumor’s venous drainage is clamped and the source of catecholamines is removed, the patient can experience profound hypotension. The anesthesia team must be ready to support blood pressure with fluids and vasopressors. Invasive monitoring with arterial lines is standard to provide beat-to-beat blood pressure measurements.
For the minority of patients with malignant pheochromocytoma (metastatic disease), surgery may not be curative but can still play a role in reducing the tumor burden and alleviating symptoms. Management of metastatic disease is multidisciplinary, involving oncology, endocrinology, and nuclear medicine. Treatments may include systemic chemotherapy, targeted molecular therapies (such as tyrosine kinase inhibitors), and radionuclide therapy. Specifically, high-dose I-131 MIBG therapy or Lutathera (Lu-177 DOTATATE) can target and destroy catecholamine-secreting cells throughout the body. The goal in these cases is to control tumor growth and manage hormonal symptoms to maintain quality of life.
The immediate postoperative period requires close monitoring in an intensive care or high-dependency unit. The sudden withdrawal of high catecholamine levels can lead to hypotension and hypoglycemia (low blood sugar). Fluid resuscitation is often needed to fill the dilated vascular bed. Physicians monitor blood glucose levels closely, as the suppression of insulin is lifted. Most patients see a normalization of blood pressure immediately or within a few weeks. However, some may remain hypertensive due to underlying essential hypertension or vascular remodeling, requiring ongoing but reduced medication.
Even after a “curative” surgery, long-term follow-up is essential. There is a risk of recurrence, either in the remaining adrenal tissue (if a partial adrenalectomy was done) or as a new tumor in the contralateral gland, especially in hereditary cases. Furthermore, metastatic disease can present years after the initial diagnosis. Standard protocol involves biochemical testing (plasma or urine metanephrines) at 2-6 weeks post-surgery to confirm success, and then annually for life. For patients with genetic syndromes, lifelong surveillance is mandatory to detect not only recurrent pheochromocytomas but also other associated tumors.
Send us all your questions or requests, and our expert team will assist you.
Medication is needed to block the effects of the hormones so that your blood pressure doesn’t spike dangerously when the surgeon touches the tumor.
Taking a beta-blocker first can cause a dangerous spike in blood pressure because it blocks the blood vessels’ ability to relax, leaving them only able to constrict.
Laparoscopy is preferred for smaller tumors, but very large or invasive tumors usually require open surgery to ensure they are removed safely and completely.
For many patients, blood pressure normalizes quickly, but some may experience low blood pressure initially or have residual high blood pressure requiring management.
You will typically need a check-up a few weeks after surgery and then annual blood or urine tests for life to ensure the tumor hasn’t returned.
Leave your phone number and our medical team will call you back to discuss your healthcare needs and answer all your questions.
Your Comparison List (you must select at least 2 packages)
